New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications.
New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications.
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DOI：
10.1111/petr.12076
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发表时间：
2013-06
影响因子：
1.3
通讯作者：
Avner ED
中科院分区：
文献类型：
--
作者：
Telega G;Cronin D;Avner ED
改进的新生儿医疗护理和肾脏替代技术提高了常染色体隐性遗传性多囊肾病(ARPKD)患者的长期存活率。据报道，那些在生命第一年幸存下来的人的十年存活率为82%，而且还在继续改善。然而，尽管总体存活率增加，系统性高血压和肾脏疾病的其他并发症的治疗有所改善，但近50%的幸存者将在生命的第一个十年内发展为终末期肾脏疾病(ESRD)。除肾脏病理外，ARPKD患者还会出现胆管板畸形，肝内外胆管囊状扩张，导致先天性肝纤维化(CHF)和Caroli综合征。许多CHF患者会发展为门脉高压，导致食道静脉曲张、脾肿大、脾功能亢进、蛋白失调性肠病和胃肠道出血。门静脉高压症的治疗可能需要内窥镜下食道静脉曲张套扎术或门体分流术。肝脏受累的并发症可能包括上行性胆管炎、胆汁淤积和脂溶性维生素吸收不良，以及罕见的良性或恶性肝脏肿瘤。ARPKD患者最终发展为终末期肾脏疾病，并最终需要肾移植，他们会出现一系列与潜在肝胆疾病相关的独特并发症。在这篇综述中，我们着重于针对这些具有挑战性的患者的新方法，包括ARPKD患者等待肾移植的严重慢性肾脏疾病患者的肝移植适应证。虽然ARPKD和孤立肾移植患者的存活率与年龄匹配的因其他原发肾脏疾病而接受肾移植的儿童患者相当，但-80%的ARPKD肾移植患者的死亡归因于胆管炎/脓毒症，这与他们的肝胆疾病有关。最近的数据显示，儿童肝移植受者的手术死亡率在1年时降至不到10%。肾移植受者使用的免疫抑制方案对大多数肝移植受者来说是足够的。因此，我们建议，在有反复胆管炎或门脉高压并发症的ARPKD患者中，肝肾联合移植是一种可行的选择。虽然还需要进一步的研究来证实我们的方法，但我们相信，肝肾联合移植可能会降低精心挑选的伴有终末期肾病和临床意义的CHF的ARPKD患者的总体死亡率和发病率。
Improved neonatal medical care and renal replacement technology has improved the long term survival of patients with autosomal recessive polycystic kidney disease (ARPKD). Ten year survival of those surviving the first year of life is reported to be 82% and is continuing to improve further. However, despite increases in overall survival and improved treatment of systemic hypertension and other complications of their renal disease, nearly 50% of survivors will develop end stage renal disease (ESRD) within the first decade of life . In addition to renal pathology, patients with ARPKD develop ductal plate malformations with cystic dilation of intra- and extra-hepatic bile ducts resulting in congenital hepatic fibrosis (CHF) and Caroli syndrome. Many patients with CHF will develop portal hypertension with resulting esophageal varices, splenomegaly, hypersplenism, protein loosing enteropathy and gastrointestinal bleeding. Management of portal hypertension may require endoscopic band ligation of esophageal varices or porto-systemic shunting. Complications of hepatic involvement can include ascending cholangitis, cholestasis with malabsorption of fat soluble vitamins, and rarely benign or malignant liver tumors. Patients with ARPKD who eventually reach end-stage renal disease, and ultimately require kidney transplantation, present a unique set of complications related to their underlying hepato-biliary disease. In this review, we focus on new approaches to these challenging patients, including the indications for liver transplantation in ARPKD patients with severe chronic kidney disease awaiting kidney transplant. While survival in patients with ARPKD and isolated kidney transplant is comparable to that of age-matched pediatric patients who have received kidney transplants due to other primary renal diseases, 64–80% of the mortality occurring in ARPKD kidney transplant patients is attributed to cholangitis/sepsis which is related to their hepato-biliary disease Recent data demonstrate that surgical mortality among pediatric liver transplant recipients is decreased to less than 10% at 1 year. The immunosuppressive regimen used for kidney transplant recipients is adequate for most liver transplant recipients. We therefore suggest that in a select group of ARPKD patients with recurrent cholangitis or complications of portal hypertension, combined liver-kidney transplant is a viable option. Although further study is necessary to confirm our approach, we believe that combined liver-kidney transplantation can potentially decrease overall mortality and morbidity in carefully selected ARPKD patients with ESRD and clinically significant CHF.
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